Abstract
摘要:胚胎學上在大動脈旋轉之後,冠狀動脈才鑽入主動脈。我們根據大動脈短軸旋轉不同之角度,提出了六種冠狀動脈主要類型,可以用來在旋轉360o 各種不同的先天性心臟病之冠狀動脈分類。在主動脈造影發現大血管旋轉不但有短軸旋轉,而且有長軸旋轉。短軸旋轉與瓣膜接合處附近之冠狀動脈(Juxtacommissural origin of the coronary artery, JOCA)有關,長軸旋轉則與高位冠狀動脈有關。簡言之,三條冠狀動脈的其中兩條及另一條分別鑽入面對肺動脈的兩個伏氏竇或其上的升主動脈,排列組合只有六種可能性,這也在上述研究之實際病例中得到佐證。我們擬於未來三年的研究當中,將這些應用到正常心臟和後天性、其他先天性心臟病的冠狀動脈之分類,期了解其致病機轉;希望可以解決過去各種眾說紛云而且混淆之冠狀動脈分類法, 並據以決定術式。我們 1995 年在JACC 發表第一篇論文以來,一共發表九篇論文證實大動脈長短軸旋轉與冠狀動脈類型在不同疾病之關聯。這些研究是以心導管主動脈根部攝影為主要依據。目前電腦斷層已可辨認大動脈旋轉角度及冠狀動脈起源,如第七篇論文所述。未來第一年研究我們擬以在76 例右型大動脈完全轉位、517 例法洛氏四重症、62 例先天性已矯正之大動脈轉位以及10 例解剖上已矯正的大動脈異位整理其主動脈肺動脈幹短軸向後旋轉之特殊冠狀動脈類型與其旋轉角度,探討可否據以決定術式(跨環式右心室出口切開)。第二年研究擬以5000 例冠心症心導管檢查有異常冠狀動脈類型的病例佐以電腦斷層,探討其病發率及其與大動脈長短軸旋轉之關聯,主動脈在右後方的特殊冠狀動脈類型。第三年研究擬以大約90 例右心室雙出口病例觀察其大動脈旋轉與冠狀動脈類型,並研究大動脈向後短軸旋轉與室膈缺損位置及採用手術方法之關聯。第一年研究擬達成下列目標:(1) 這些病例的伏氏竇類型之分佈如何?主動脈肺動脈幹旋轉角度多少? 大動脈向後短軸旋轉有否特殊伏氏竇類型?(2) 這些病例的冠狀動脈類型之分佈如何?大動脈向後短軸旋轉是否伴有特殊冠狀動脈類型?(3) 是否所有主動脈根部旋轉到肺動脈幹後方的冠狀動脈類型之先天性心臟病皆可施行傳統的跨環式右心室出口重建手術。以上為第一年之研究:1.大動脈短軸向後旋轉與冠狀動脈類型與傳統的跨環式右心室出口切開之研究。第二年至第三年之研究題目如下:2.根據主動脈肺動脈幹旋轉角度推定冠心症異常冠狀動脈類型。3 右心室雙出口大動脈長短軸旋轉對冠狀動脈類型、室膈缺損位置與採用術式之關聯。
Abstract: Our research in the past is mainly involved in the evolution of coronary artery (CA) pattern invarious congenital cardiac defects and its implications in surgical management. Embryologically, the CAdevelops after septation of the aortopulmonary trunk and pierces the aortic sinus at the nearest site afteraortopulmonary rotation (APR). We found that the CA pattern is statistically dependent on the APR, andthus proposed a new categorization scheme of six basic types based on the aortic root rotation. Since thereare only 3, if one CA entering one facing sinus and the other two CA’s entering the other; randomlyspeaking, there would be only six possibilities for the basic arrangement of CA’s (not including the singleCA and its variant), because only three main CA’s exist and usually they penetrate either of the aorticsinus facing the pulmonary trunk. We also found that, in addition to the short-axis rotation, which isrelated to the juxtacommissural origin of the CA, there is also a long-axis rotation, which is related tohigh takeoff of the CA. On the basis of these findings, we have proposed appropriate diagnostic andsurgical techniques to manage different CA patterns in patients. In the coming three years, we will applyour categorization of CA pattern to normal hearts and acquire or other congenital cardiac defects.Hopefully the present the complex CA classification of all the normal and abnormal hearts can besimplified and unified to our categorization according to the APR, and choose the surgical options toavoid CA injury accordingly.In the first year, we will identify CA types and APR in transposition of the great arteries (n = 76),tetralogy of Fallot (n = 517), congenital corrected transposition (n = 62), and anatomical correctedmalposition (ACM) (n = 10). Specific aortic sinus pattern with the posterior APR more than right or leftlateral 90 will be identified. The concomitant CA pattern will be safe to perform an anterior transannularincision to relieve their pulmonary outflow obstruction. In the second year, we will evaluate the unusualCA. patterns in around 5000 coronary artery disease patients underwent coronary angiograms by CT. Therelation of specific CA pattern to posterior APR will be verified and its incidence revealed. In the thirdyear, the implications of long/short axis APR in 90 cases of double outlet right ventricle to the locationventricular septal defect and CA pattern will be stratified to the pathogenesis and surgical options.Three-year study includes the following:1. Coronary artery patterns in congenital heart defects with posterior located aorta and its implication ontransannular pulmonary outflow incision2. The implications of the long/short axis aortopulmonary rotation to the abnormal coronary arterypatterns in coronary heart disease3. The influence of the long/short axis aortopulmonary rotation to the location of ventricular septal defectand coronary pattern in double outlet right ventricle and its implication on surgical options
Keyword(s)
冠狀動脈類型
法洛氏四重症
大動脈轉/異位
先天已矯正之大動脈轉位
解剖上已矯正的大動脈異位
大動脈短軸旋轉
coronary artery pattern
tetralogy of Fallot
transposition of the great arteries
congenitally corrected transposition of the great arteries
anatomically corrected malposition of the great arteries
short axis aortopulmonary rotation.